Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare, but well documented entity. We report a lady who presented with progressive quadriparesis, with cervical extradural lesion on magnetic resonance imaging. She underwent decompression of the lesion and histological diagnosis of the lesion was Rosai-Dorfman disease. On one-year follow-up, she had complete improvement of the deficits with no further progression of the lesion. The presentation of this disease as an isolated spinal extradural mass lesion is quite rare, with only six cases reported in literature.